Kimura's disease: a diagnostic and therapeutic challenge.

INTRODUCTION: Kimura's disease (KD) is a rare, benign, chronic inflammatory disease with unknown aetiology. Its manifestation is protean. KD has a predilection for the head and neck area, and typically presents as tumour-like lesions that could be easily misdiagnosed. We review our experience with four recent cases. METHODS: Over a four-year period, all patients admitted to Singapore General Hospital with KD of the head and neck region were retrospectively reviewed. Biodata, presenting symptoms and clinical parameters, especially serum eosinophil levels, preoperative investigations, type of surgical procedures and outcome were documented. RESULTS: Four patients presented with KD of the head and neck and displayed varied manifestations of the disease. All the patients had raised serum eosinophil levels. None of them had renal involvement. Preoperative computed tomography were performed in two of the patients and showed features suggestive of KD. Fine-needle aspiration cytology that was performed in two patients was not useful in the diagnosis. All the patients underwent surgical excision of the lesions. Only one patient had multiple recurrence, both at the original and remote sites in the head and neck. CONCLUSION: The clinical presentation and behaviour of KD is very variable. Preoperative imaging is useful in the diagnosis of the disease but the final diagnosis is histological. Surgical excision is the current treatment of choice but recurrence is common. A high index of suspicion and awareness is vital in the early diagnosis and management of KD.

A clinical review of granulosa cell tumours of the ovary cases in KKH.

INTRODUCTION: Granulosa cell tumour (GCT) represents the largest group of sex-cord stromal tumours and comprises 1.5-3% of ovarian malignancy. The aim is to determine the incidence of the disease, study the profile of local patients, and assess the use of imaging studies in the diagnosis of the tumour. MATERIAL AND METHODS: Clinical records of 19 patients diagnosed with GCT between October 1988 and July 1997 in Kandang Kerbau Hospital (KKH) were reviewed. RESULTS: GCT accounts for 3.5% of ovarian malignancy (54 out of 1552) in Singapore, of which 94.7% are adult GCT. In our study, patients are mainly peri/postmenopausal women (63.2%) in their 50s who experience post-menopausal bleeding. There is a high incidence of association with endometrial hyperplasia (40%). Ultrasound scans are able to predict the size and involvement of the tumour rather accurately. In our study sample, 13 patients (68.4%) presented with Stage 1 of the disease, none with Stage 2, 1 with Stage 3 (5.3%) and none with Stage 4. The other 5 patients (26.3%) were unstaged. Only one patient required adjuvant chemotherapy. CONCLUSION: The local data with regards to GCT is congruent with those found in foreign literature. However, in our study, there were no patients with recurrence whereas GCTs are known to be late recurring in up to 20% of patients 10-20 years after diagnosis. This is probably attributed to the relatively short period of follow-up in this study. Thus, despite the fact that there is no evidence of recurrence of disease in our current study, we still recommend a vigilant follow-up protocol on all patients as literature has proven that with early detection of recurrences, it is possible to achieve complete cure.

Primary ovarian pregnancy: current diagnosis and management.

We reviewed five cases of ovarian pregnancy that were diagnosed at the Kandang Kerbau Hospital Histopathology Laboratory over three years. The clinical presentation, diagnosis and management of these patients are discussed in detail. This condition usually occurs in parous fertile women as evidenced by three of the five patients studied. It is probably an accidental event with no predisposing features as compared to the tubal pregnancy patient. The diagnosis has been aided by the recent advances in human chorionic gonadotrophin determination and ultrasound. Ultrasound, especially transvaginal ultrasound scanning has proven to be an invaluable tool in the diagnosis of this condition. Fertility after conservative surgical procedures does not appear to be affected and ovarian wedge resection or ovarian cystectomy is the treatment of choice.

A case of localised ureteric amyloidosis.

We describe a case of localised amyloidosis of the distal ureter in a 66-year-old lady who presented with intermittent haematuria. Ureteric transitional cell carcinoma was strongly suspected from the clinical and radiological features, and subsequent nephroureterectomy was performed. Histology revealed nodular eosinophilic deposits in the ureteric wall, with the staining characteristics of amyloid. The literature is reviewed, and conservative surgery is advocated, based on a greater awareness together with improved intraoperative diagnosis of this rare lesion.

Primary unruptured ovarian pregnancy--a case report.

A rare case of an unruptured ovarian ectopic pregnancy is reported. This patient had a dilatation and curettage for an incomplete abortion and no products of conception were revealed in the histology specimen. A repeat ultrasound scan detected an ectopic pregnancy. Laparotomy confirmed the diagnosis of an unruptured ovarian pregnancy. The problems with the diagnosis and the management of this case are discussed.

Primary extrauterine endometrial stromal neoplasms: a clinicopathologic study of 20 cases and a review of the literature.

We present the results of a clinicopathologic study of 20 patients with primary extrauterine endometrial stromal sarcoma (ESS). The sites of the primary neoplasm and the number of patients with sufficient follow-up for survival analysis are as follows: ovary (three of four), fallopian tube (one of one), pelvic cavity (six of eight), abdominal cavity (five of six), and retroperitoneum (one of one). Evaluation of all patients included the mitotic index (MI) and cytologic atypia. Thirteen of the sixteen patients eligible for survival analysis had tumors with an MI < 10 and would be classified as low-grade stromal sarcomas in the Norris and Taylor scheme. Eight (62%) of the 13 had one or more relapses; of these, three died of disease at 35, 108, and 120 months, respectively, and another patient was alive with disease at 96 months. The other four patients who were treated after a relapse showed no evidence of disease after relapse at 36, 57, 63, and 146 months, respectively. Two of the 13 patients had tumor considered unresectable at the time of diagnosis; both died of disease at 5 and 10 months, respectively. Neither MI nor cytologic atypia were predictive of tumor recurrence or death from tumor. We also extracted clinical and morphologic data from all previous reports of primary extrauterine ESS, combined them with our 20 patients, and then compared the combined group with 17 cases of primary high-stage uterine ESS we presented in an earlier report. Not surprisingly, the behavior of the primary extrauterine ESS was more reminiscent of high-stage primary uterine ESS than low-stage primary uterine ESS.

Benign teratoma of the fallopian tube: a case report.

A benign teratoma of the fallopian tube in a 33-year-old woman with primary subfertility is reported. This is the first case reported in Singapore. The features of benign teratoma of the fallopian tube is discussed.

Fine needle aspiration of 123 head and neck masses--an initial experience.

Fine needle aspiration (FNA) is a relatively new technique in the investigation of head and neck masses in Singapore. We present our initial experience with 123 neck masses; 82 were lymph nodes, 33 were salivary gland masses and eight were others. The inadequacy rate (percentage of hypocellular yields) was 12.2% with a cytology: histology accuracy rate of 82.6%. The aspiration trolley set-up and improvements in tissue handling reduced the number of inadequate smears. A study of nasopharyngeal carcinoma (NPC) with neck recurrence showed the difficulty encountered with FNA in irradiated necks. In "occult" (clinically normal nasopharynx) NPC with a neck mass as the first presentation, FNA guided us to re-search the nasopharynx.

Embryonal carcinoma arising in Turner's syndrome.

Risk of malignant transformation in a dysgenetic gonad is high. This is a report of a 23 year-old female who presented with symptoms and signs of ovarian neoplasm which was histologically confirmed as embryonal carcinoma. She had features of Turner's syndrome and was found to have XO chromosomal constitution. Embryonal carcinoma arising in a dysgenetic gonad is uncommon. It is even rarer when it arises in a patient with pure 45 XO. The following is a case report which highlights the role of chemotherapy and surgery in the management. It includes a literature review on the clinical features, genetic variants and malignant transformation in dysgenetic gonad. The role of prophylactic removal of dysgenetic gonad is discussed.

Patterns and implications of subclinical vulval human papillomavirus infection: the impact of PCR analysis.

This study was performed to elucidate the presence of human papillomavirus (HPV) infection of the vulva by colposcopy, histology and the polymerase chain reaction (PCR). Colposcopy defined 5 patterns of vulval epithelial lesions inconspicuous to the naked eye. Of these 75 subclinical vulval lesions, HPV infection was diagnosed by histology in 20.0% of minor epithelial changes with faint acetowhitening, 52.2% of conspicuous acetowhite lesions, 63.0% of acetowhite areas with satellite lesions, 84.6% of villous lesions, and 85.7% of villous lesions with surrounding acetowhitening. The corresponding HPV DNA positivity rates by PCR were 60.0%, 73.9%, 70.4%, 84.6% and 100% respectively. The oncogenic HPV type 16 was detected by PCR in 37.3% of the samples. These results provide firm evidence for the prevalent existence of subclinical HPV lesions of the vulva. Some of these infections may not produce significant morphologic changes detectable by colposcopy or histology. Subclinical vulval lesions are common and may constitute a reservoir for repeated cervical HPV infections, as well as a source of contamination of cervical samples for HPV DNA detection by sensitive molecular techniques.